title: The fasting test in paediatrics: application to the diagnosis of pathological hypo- and hyperketotic states.
abstract: A 24-h fasting test was performed in 48 control children, in 9 hypoketotic patients with inherited defects of fatty acid oxidation and in 2 hyperketotic patients with inherited defects of ketolysis. The control group was then divided into three age groups on the basis of different adaptation to fasting. Concentrations of blood glucose, lactate, free fatty acids (FFA), 3-hydroxybutyrate, acetoacetate and carnitine were measured after 15 h, 20 h and 24 h of fasting. Significant negative correlations were found in the control group between plasma total ketone bodies (KB) and plasma glucose (P less than 0.001), plasma carnitine (P less than 0.005) and the amplitude of glycaemic response to glucagon at the end of the fast (P less than 0.01). FFA/KB ratio and the product of final fasting values of glucose and ketones were useful to differentiate between hypoketotic or hyperketotic patients and normal subjects. In children with a suspected or definite hyperketotic or hypoketotic disorder, a fasting test must only be performed in healthy patients, in good nutritional condition with non-diagnostic basal biochemical investigations. Carefully supervised fasting should be continued sufficiently to allow ketogenesis and ketolysis to become activated.
authors.affiliation: Département de Pédiatrie, Inserm U-12, Hôpital des Enfants-Malades, Paris, France.
authors.forename: J P
authors.initials: JP
authors.lastname: Bonnefont
authors.forename: N B
authors.initials: NB
authors.lastname: Specola
authors.forename: A
authors.initials: A
authors.lastname: Vassault
authors.forename: A
authors.initials: A
authors.lastname: Lombes
authors.forename: H
authors.initials: H
authors.lastname: Ogier
authors.forename: J B
authors.initials: JB
authors.lastname: de Klerk
authors.forename: A
authors.initials: A
authors.lastname: Munnich
authors.forename: M
authors.initials: M
authors.lastname: Coude
authors.forename: M
authors.initials: M
authors.lastname: Paturneau-Jouas
authors.forename: J M
authors.initials: JM
authors.lastname: Saudubray
mesh_terms: D020155:3-Hydroxybutyric Acid; D000293:Adolescent; D001786:Blood Glucose / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D005215:Fasting / Q000097:blood*; D005230:Fatty Acids, Nonesterified / Q000097:blood; D005260:Female; D006801:Humans; D006885:Hydroxybutyrates / Q000097:blood; D007003:Hypoglycemia / Q000097:blood; D007223:Infant; D007657:Ketone Bodies / Q000097:blood*; D008052:Lipid Metabolism, Inborn Errors / Q000175:diagnosis; D008297:Male
chemical_list: D001786:Blood Glucose; D005230:Fatty Acids, Nonesterified; D006885:Hydroxybutyrates; D007657:Ketone Bodies; D020155:3-Hydroxybutyric Acid